Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing |
Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. |
Purpose : This study aims to examine and compare the features of rolandic epilepsy. Methods : Of 158 patients selected retrospectively, 116 had typical (group A) and 42 had atypical (group B) rolandic epilepsy, as defined by Worrall's criteria. Results : The age at onset of the seizures in group A was 8.6¡¾2.0 y and 6.2¡¾1.7 y in group B... |
Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial... |
Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease.... |
Purpose : To identify clinical and laboratory features of atypical Kawasaki disease(KD), and to develop criteria for early diagnosis of atypical KD patients. Methods : All patients with KD treated at our hospital from January 1998 to June 2000 were reviewed retrospectively. Results : Among a total of 167 patients, 28(16.8%) were atypical KD of which seven(25%) were infants. Among the... |
Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence... |
Purpose : Kawasaki disease(KD) is known as one of the most important causes of acquired heart disease in children. But the incidence of acquired heart disease can be reduced by early diagnosis and treatment with large amounts of intravenous γ-globulin(IVGG). For early diagnosis and treatment of atypical KD, we analyzed and compared the clinical features, laboratory findings and coronary lesions in patients with typical... |
Purpose : We tried to classify the different type of CLD and assess the risk factors for classical CLD and atypical CLD. Methods : Retrospective cohort analysis was done in 120 preterm infants with birth weights less than 1,500 g who were admitted to NICU in Seoul National University Children's Hospital between Jan. 1993 and Dec. 1998 and survived more... |
We report a case of atypical benign partial childhood epilepsy in a 11 years old male child whose case has been followed up for 6 years. His first symptom was focal seizure of the left side of his face during a drowsy state, followed by focal seizures of left fingers and legs. At that time he had been on phenobarbital... |
Purpose : Without a specific diagnostic method, the clinical diagnostic criteria for atypical Kawasaki disease is known as the presence of coronary artery changes in a patient who did not meet the already estabilished clinical criteria. With this criteria, we are put into dilemma because atypical Kawasaki disease should exhibit coronary artery disease. So, we demonstrated the rate of coronary artery involvement in atypical... |
Joubert syndrome is frequently associated with developmental delay and mental retardation, neonatal tachypnea and apnea, abnormal eye movements, and ataxia. It is inherited by an autosomal recessive trait. The most significant and constant neuropathological finding is partial or total agenesis of cerebellar vermis. Absence of the vermis results in a triangular-shaped mid-fourth ventricle and a "bat-wing" shaped fourth ventricle superiorly. The superior cerebellar peduncles... |
Kawasaki disease is an acute febrile illness of unknown etiology that occurs predominantly in infants and early childhood. It had aroused intense interest because of the sequelae of coronary arteritis accompanied by coronary artery aneurysms and thrombotic occlusion, which may lead to ischemic heart disease or sudden death. Atypical Kawasaki disease is coined to describe patients who have coronary abnormalities,... |
Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4 deficiency in brother and sister, confirmed by biochemical study of blood and urine. They had suffered from a progressive neurologic illness such as mental retardation, severe hypotonia, seizure, and athetotic movements... |
Patients with atypical or incomplete Kawasaki disease are at same risk for development of coronary artery complications as typical Kawasaki disease. In this communication we report six patients with unusual presentation of Kawasaki disease complicated by coronary artery aneurysms, in whom correct diagnosis were not made in time for proper treatment. One of these patients died from massive myocardial ischemia... |
Kawasaki disease is described by fever lasting five days or more, bilateral conjunctival injection, changes of lips and oral cavity, polymorphous exanthema, acute non-purulent cervical lymphadenopathy, and changes of extremities. Atypical Kawasaki disease is defined as fewer than 4 of 6 criteria described above including coronary artery abnormalities. Especially, atypical clinical manifestations of Kawasaki disease appear in infants younger than 6... |
Form March 1982 to December 1991, 110 cases of typical post-streptococcal glomerulonephritis and 25 cases of Atypical acute glomerulonephritis were clinically investigated at Department of pediatrics, Kyung Hee University Hospital, and follwing Data were obtained. 1) Male to Femal ratio was 1.8:1 (Atypical AGN). Peak incidence was from 7 to 12 years. 2) The most common preceding infections were upper respiratory infection,... |
Almost of all patients with neurofibromatosis type I show caf6-au-lait macules on their skin. These macules are thought to be early sign of neurofibromatosis in childhood. We experienced a case of neurofibromatosis type I in a 10-year-old-boy who did not have caf6-au-lait macules, but progressive psychomotor retardation, short attention span, hyperactivity and megalencephaly. The brain C-T findings showed diffuse cerebromalacia and ventriculomegaly. We confirmed the... |